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  • Open Access


    Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

    Shota Kawai1, Takashi Kido1,*, Yuta Teguri1, Koji Miwa1, Tomomitsu Kanaya1, Yoichiro Ishii2, Hisaaki Aoki2, Futoshi Kayatani2, Sanae Tsumura1

    Congenital Heart Disease, Vol.18, No.4, pp. 399-411, 2023, DOI:10.32604/chd.2023.042243

    Abstract Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-to-pulmonary artery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed all patients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January 2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiring early modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonary blood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank: p = 0.04), but the statistical significance… More >

  • Open Access


    Associations between Bodyweight and Clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review

    Emma Payne1,2, Thomas Wilson2,3, Marjan Haghighi1,4, Zoe McCallum2,3, Yves d’Udekem5, Julian Ayer1,4,*

    Congenital Heart Disease, Vol.17, No.6, pp. 617-639, 2022, DOI:10.32604/chd.2022.024775

    Abstract Background: Patients born with a single ventricle circulation commonly experience growth failure in early life, which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectory and clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, a systematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patients with clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluated by Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies. Results: Of 527 studies that underwent title… More >

  • Open Access


    Preoperative Feeding in Single Ventricle Neonates is Predictive of Shorter Time to Goal Feed

    Alyssia Venna1, Kathleen Reid2, Sarah Davis2, Jiaxiang Gai3, Yves d’Udekem1, Sarah Clauss2,*

    Congenital Heart Disease, Vol.17, No.5, pp. 505-518, 2022, DOI:10.32604/chd.2022.021571

    Abstract Background: Patients with single ventricle anatomy are at increased risk of growth failure and malnutrition. Amongst cardiac centers, there is little standardization of feeding practices in this complex population. We hypothesized that initiation of our center’s preoperative feeding protocol would result in decreased gastrostomy tube (G-tube) use, decreased length of stay and would not result in increased Necrotizing Enterocolitis (NEC) rates. Methods: A single institution review of 52 patients who had undergone stage I single ventricle palliative repair was performed. Patient diagnoses were hypoplastic left heart syndrome (39%), atrioventricular canal (15%), and other (46%). Postoperative parameters such as time to… More > Graphic Abstract

    Preoperative Feeding in Single Ventricle Neonates is Predictive of Shorter Time to Goal Feed

  • Open Access


    A Ring-Reinforced Right Ventricle to Pulmonary Artery Conduit is Associated with Better Regional Mechanics after Stage I Norwood Operation

    Benjamin Zielonka1,2,*, David M. Harrild1,2, Sunil J. Ghelani1,2, Eleni G. Elia1,2, Christopher W. Baird3,4, Andrew J. Powell1,2, Rahul H. Rathod1,2

    Congenital Heart Disease, Vol.17, No.5, pp. 591-603, 2022, DOI:10.32604/chd.2022.021509

    Abstract Background: The right ventricle to pulmonary artery conduit (RVPAC) may impair right ventricular (RV) function in patients with functional single right ventricles. Modification of the RVPAC using a ring-reinforced end with dunked insertion into the RV through a limited ventriculotomy may reduce the impact on RV function. We compared RV segmental strain between patients with a traditional RVPAC and ring-reinforced RVPAC using feature tracking cardiovascular magnetic resonance (CMR) imaging. Methods: Patients with CMR examinations after Stage I operation with RVPAC between 2000 and 2018 were reviewed. Ventricular mass, volumes, late gadolinium enhancement (LGE), and peak radial and circumferential strain of… More >

  • Open Access


    Comparison of Intracardiac and Extracardiac Malformations Associated with Single Atrium, Single Ventricle and Single Atrium-Single Ventricle Using DualSource Computed Tomography

    Tong Pang#, Li Jiang#, Yi Zhang, Mengxi Yang, Jin Wang, Yuan Li*, Zhigang Yang*

    Congenital Heart Disease, Vol.17, No.4, pp. 479-489, 2022, DOI:10.32604/chd.2022.020401

    Abstract Background: To evaluate the qualitative and quantitative differences between intracardiac and extracardiac vascular malformations in patients with a single atrium (SA), single ventricle (SV) and single atrium-single ventricle (SA-SV) using dual-source CT (DSCT), and to compare the diagnostic performances of DSCT and transthoracic echocardiography (TTE). Methods: This retrospective study included 24 SA, 75 SV and 24 SA-SV patients who underwent both DSCT and TTE before surgery. The diagnostic values of DSCT and TTE for intracardiac and extracardiac malformations were compared according to the surgical results. The diameters of the major artery and vein were measured and calculated based on DSCT… More >

  • Open Access


    Effectiveness of Bilateral Pulmonary Artery Banding in Patients with Hypoplastic Left Heart Syndrome and Congenital Heart Defects with A Functional Single Ventricle: A Single-Center Retrospective Study

    Vitaliy Suvorov1,*, Vladimir Zaitcev1, Karolina Andrzejczyk2

    Congenital Heart Disease, Vol.17, No.3, pp. 365-374, 2022, DOI:10.32604/chd.2022.019126

    Abstract Background: Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome (HLHS) and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period, especially in severely unstable patients. In our study we compared different surgical techniques of bilateral pulmonary artery banding (PAB) in respect to their success in balancing systemic and pulmonary blood flow. Methods: We included 44 neonates with a HLHS and congenital heart diseases (CHD) with a functional single ventricle underwent a hybrid operation: bilateral PAB and patent ductus arteriosus stenting. The hybrid… More >

  • Open Access


    Multi-Institutional US Experience of the Occlutech© AFR Device in Congenital and Acquired Heart Disease

    Barry O’Callaghan1, Jenny Zablah1, Joseph Vettukattil2, Daniel Levi3,4, Morris Salem4, Allison Cabalka5, Jason Anderson6, Makram Ebeid6, Ryan Alexy7, Gareth J. Morgan1,*

    Congenital Heart Disease, Vol.17, No.1, pp. 107-116, 2022, DOI:10.32604/CHD.2022.018590

    Abstract Objectives: To detail the US multi-institutional experience with the Occlutech© (Occlutech International AB, Helsingborg, Sweden) atrial flow regulator (AFR) in children and adults with acquired or congenital heart disease. Background: The creation of a long-term atrial communication is desirable in several cardiovascular disease phenotypes, most notably pulmonary arterial hypertension, disorders of increased left ventricular filling and increased cavopulmonary pressures in patients with a Fontan type circulation. Methods: Patients were identified for inclusion from the AFR device manufacturer database. Data was collected using a RedCap database following IRB approval. 8 weeks of follow up data was sought for each patient based… More >

  • Open Access


    The Glenn Shunt Revisited, A Single Center Registry in Ain Shams University Cardiology Department

    Mina Tewfik1,2,*, Maiy El-Sayed1, Alaa Roushdy1, Soha Romeih2,3, Dina Ezzeldin1, Hebatalla Attia1

    Congenital Heart Disease, Vol.17, No.1, pp. 71-85, 2022, DOI:10.32604/CHD.2022.018372

    Abstract Background: Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is, patients unsuitable for biventricular repair. In our country they are performed at an older age than that recommended by the literature. So, we aim to assess post bidirectional Glenn shunt patients to detect the presence of any complications and to evaluate their functional capacity. Methods: This was a descriptive study that included all patients who underwent a bidirectional Glenn shunt and were referred for follow up in Ain shams university hospitals. History taking including NYHA class and physical activity, clinical examination,… More >

  • Open Access


    Natural History of Kawashima Palliation in Single-Ventricle and Interrupted Inferior Vena Cava Heart Disease in China 11 Years Result

    Yajuan Zhang, Jun Yan*, Qiang Wang, Shoujun Li, Jing Sun, Shuo Dong, Jiachen Li

    Congenital Heart Disease, Vol.16, No.4, pp. 383-392, 2021, DOI:10.32604/CHD.2021.015479

    Abstract Background: The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow (AnPBF) in single-ventricle (SV) and interrupted inferior vena cava (IVC) heart disease is still uncertain as yet. Methods: We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018, perioperative, operative and postoperative characteristics were recorded. Results: A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years (range 0.5–24.7 years), of which 12 (66.7%) were male and 6 (33.3%) were female. The mean saturation was… More >

  • Open Access


    An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

    Sarah Pradhan1,*, Eileen Broomall2, Russel Hirsch1

    Congenital Heart Disease, Vol.16, No.4, pp. 393-396, 2021, DOI:10.32604/CHD.2021.015257

    Abstract The differential diagnosis for irritability in children is broad. In patients with congenital heart disease, one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure (CVP). In patients with single-ventricle physiology, the second stage of palliation includes bidirectional Glenn, which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle. Typically, early in the post-operative period, patients may experience a headache due to the acute increase in CVP, and symptoms improve over time. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a… More >

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